Recessive loss-of-function mutations in SCN9A result in congenital insensitivity to pain (CIP), whereas gain-of-function, dominant mutations lead to sensory neuronal hyperexcitability and the development of painful phenotypes described as inherited or primary erythromelalgia (PEM or IEM) (Yang et al. 2004; Dib-Hajj et al. 2005) and paroxysmal extreme pain disorder (PEPD) (Fertleman et al. 2006; Choi et al. 2011). This evidence concerns the gene SCN9A and primary erythermalgia.